Acne Keloidalis Nuchae 

  • Author: Philip R Letada, MD; Chief Editor: William D James, MD   more...
 
Updated: Aug 5, 2011
 

Background

Acne keloidalis nuchae (AKN) is a scarring form of chronic folliculitis that manifests as follicular-based papules and pustules, which eventuates in keloidlike lesions. The lesions are most pronounced on the occipital scalp and the posterior part of the neck, and they occur almost exclusively in young males of African descent.[1] The term acne keloidalis nuchae is somewhat of a misnomer because the lesions do not occur as a result of acne vulgaris and are histologically not keloidal.[2]

Acne keloidalis nuchae was first recognized as a discrete entity in the late 1800s. Hebra was the first to describe and document this condition in 1860, under the name sycosis framboesiformis. Subsequently in 1869, Kaposi described this same condition as dermatitis papillaris capillitii.[3] The term acne keloidalis was then given to this condition in 1872 by Bazin, and, since that time, this is the name most often used in the literature.[2]

Clinically, the lesions initially manifest as mildly pruritic follicular-based papules and pustules on the nape of the neck. Because the folliculitis is persistent, ultimately keloidlike plaques eventuate. The area is typically hairless, but broken hair shafts, tufted hairs, and ingrown hairs can sometimes be identified within and at the margins of the plaques. Over time, the plaques typically slowly expand. The lesions are disfiguring and can be painful. Abscesses and sinus tracts with purulent discharge may develop in advanced cases. Comedones are not a common feature of acne keloidalis nuchae.

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Pathophysiology

The exact etiology of acne keloidalis nuchae (AKN) remains obscure; however, one postulation is that chronic irritation and inward growth of coarse, curved hairs may play a role in the development of these lesions. This hypothesis is supported by the fact that lesions are exacerbated by close shaving and/or recurrent rubbing of the area by clothes or athletic gear. In a study of 453 high school, college, and professional American football players, 13.6% of African American athletes had acne keloidalis nuchae, whereas none of the white athletes had acne keloidalis nuchae.[4]

Similar to pseudofolliculitis barbae, a condition that also occurs more commonly in African Americans, some have proposed that close shaving or shearing of coarse, curved hairs facilitates the reentry of the free end of the hair into the skin, which then invokes an acute inflammatory response. Men who have haircuts more frequently than once a month are at higher risk of developing acne keloidalis nuchae.[5]

Although the ingrowing hairs account for small papules, they are not sufficient to explain the progressive scarring alopecia that occurs in some patients. Patients with progressive scarring alopecia often exhibit recurrent crops of small pustules and may have a condition akin to folliculitis decalvans. Chronic low-grade bacterial infection, autoimmunity, and some types of medication (eg, cyclosporine, diphenylhydantoin, carbamazepine) have also been implicated in the pathogenesis in some patients.[6, 7]

Sperling et al classify acne keloidalis nuchae as a primary form of inflammatory scarring alopecia and suggest that overgrowth of microorganisms does not play an essential role in the pathogenesis of acne keloidalis nuchae. They also found no association between pseudofolliculitis barbae and acne keloidalis nuchae.[8]

After extensive histological and ultrastructural studies of acne keloidalis nuchae lesions, Herzberg et al proposed that a series of events must happen in order for acne keloidalis nuchae to occur, namely the following[9] :

  1. The initial process begins as acute perifollicular inflammation followed by weakening of the follicular wall at the level of the lower infundibulum, the isthmus, or both.
  2. The naked hair shaft is then released into the surrounding dermis, which acts as a foreign body and incites further acute and chronic granulomatous inflammation. This process is clinically manifested by small follicular-based papules and pustules.
  3. Subsequently, fibroblasts deposit new collagen and fibrosis ensues.
  4. Distortion and occlusion of the follicular lumen by the fibrosis results in retention of the hair shaft in the inferior aspect of the follicle, thereby perpetuating the granulomatous inflammation and scarring. This stage is marked by plaques of hypertropic scar.
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Epidemiology

Frequency

United States

Acne keloidalis nuchae is said to represent 0.45% of all dermatoses affecting black persons.

Mortality/Morbidity

The plaques of acne keloidalis nuchae slowly expand over time, and, although medically benign, acne keloidalis nuchae can be a psychologically devastating condition. Chronic pruritus and drainage may occur, and, ultimately, scarring alopecia may ensue.

Race

Acne keloidalis nuchae is most prevalent in African Americans; however, it has occasionally been reported in Hispanics and Asians, and, rarely, in whites.

Sex

Although early literature inferred that acne keloidalis nuchae only affects males, it is now known to occur in females, with a male-to-female ratio of approximately 20:1.[10]

Age

Most cases occur in persons aged 14-25 years. Lesions manifesting prior to puberty or in persons older than 50 years is unusual.[8]

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Contributor Information and Disclosures
Author

Philip R Letada, MD  Resident Physician, Department of Dermatology, Naval Medical Center San Diego

Philip R Letada, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Association of Military Dermatologists

Disclosure: Nothing to disclose.

Coauthor(s)

Elizabeth Kline Satter, MD, MPH  Chairman, Department of Dermatology, Naval Medical Center San Diego

Elizabeth Kline Satter, MD, MPH is a member of the following medical societies: Alpha Omega Alpha and American Medical Women's Association

Disclosure: Nothing to disclose.

Specialty Editor Board

James W Patterson, MD  Professor of Pathology and Dermatology, Director of Dermatopathology, University of Virginia Medical Center

James W Patterson, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Dermatopathology, Royal Society of Medicine, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

John G Albertini, MD  Consulting Staff, Dermatologic Surgery, The Skin Surgery Center; Program Director, ACGME Accredited Fellowship in Procedural Dermatology

John G Albertini, MD is a member of the following medical societies: American Academy of Dermatology and American College of Mohs Micrographic Surgery and Cutaneous Oncology

Disclosure: Nothing to disclose.

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, A. Paul Kelly, MD, to the development and writing of this article.

References
  1. Dinehart SM, Herzberg AJ, Kerns BJ, Pollack SV. Acne keloidalis: a review. J Dermatol Surg Oncol. Jun 1989;15(6):642-7. [Medline].

  2. Gloster HM Jr. The surgical management of extensive cases of acne keloidalis nuchae. Arch Dermatol. Nov 2000;136(11):1376-9. [Medline].

  3. Adamson HG. Dermatitis papillaris capillittii (Kaposi). Acne keloid. Br J Dermatol. 1914;26:69-83.

  4. Knable AL Jr, Hanke CW, Gonin R. Prevalence of acne keloidalis nuchae in football players. J Am Acad Dermatol. Oct 1997;37(4):570-4. [Medline].

  5. Khumalo NP, Jessop S, Gumedze F, Ehrlich R. Hairdressing and the prevalence of scalp disease in African adults. Br J Dermatol. Nov 2007;157(5):981-8. [Medline].

  6. Grunwald MH, Ben-Dor D, Livni E, Halevy S. Acne keloidalis-like lesions on the scalp associated with antiepileptic drugs. Int J Dermatol. Oct 1990;29(8):559-61. [Medline].

  7. Wu WY, Otberg N, McElwee KJ, Shapiro J. Diagnosis and management of primary cicatricial alopecia: part II. Skinmed. Mar-Apr 2008;7(2):78-83. [Medline].

  8. Sperling LC, Homoky C, Pratt L, Sau P. Acne keloidalis is a form of primary scarring alopecia. Arch Dermatol. Apr 2000;136(4):479-84. [Medline].

  9. Herzberg AJ, Dinehart SM, Kerns BJ, Pollack SV. Acne keloidalis. Transverse microscopy, immunohistochemistry, and electron microscopy. Am J Dermatopathol. Apr 1990;12(2):109-21. [Medline].

  10. Kelly AP. Pseudofolliculitis barbae and acne keloidalis nuchae. Dermatol Clin. Oct 2003;21(4):645-53. [Medline].

  11. George AO, Akanji AO, Nduka EU, Olasode JB, Odusan O. Clinical, biochemical and morphologic features of acne keloidalis in a black population. Int J Dermatol. Oct 1993;32(10):714-6. [Medline].

  12. Goh MS, Magee J, Chong AH. Keratosis follicularis spinulosa decalvans and acne keloidalis nuchae. Australas J Dermatol. Nov 2005;46(4):257-60. [Medline].

  13. Janjua SA, Iftikhar N, Pastar Z, Hosler GA. Keratosis follicularis spinulosa decalvans associated with acne keloidalis nuchae and tufted hair folliculitis. Am J Clin Dermatol. 2008;9(2):137-40. [Medline].

  14. Luz Ramos M, Munoz-Perez MA, Pons A, Ortega M, Camacho F. Acne keloidalis nuchae and tufted hair folliculitis. Dermatology. 1997;194(1):71-3. [Medline].

  15. Adegbidi H, Atadokpede F, do Ango-Padonou F, Yedomon H. Keloid acne of the neck: epidemiological studies over 10 years. Int J Dermatol. Oct 2005;44 Suppl 1:49-50. [Medline].

  16. Quarles FN, Brody H, Badreshia S, et al. Acne keloidalis nuchae. Dermatol Ther. May-Jun 2007;20(3):128-32. [Medline].

  17. Layton AM, Yip J, Cunliffe WJ. A comparison of intralesional triamcinolone and cryosurgery in the treatment of acne keloids. Br J Dermatol. Apr 1994;130(4):498-501. [Medline].

  18. Kantor GR, Ratz JL, Wheeland RG. Treatment of acne keloidalis nuchae with carbon dioxide laser. J Am Acad Dermatol. Feb 1986;14(2 Pt 1):263-7. [Medline].

  19. Shah GK. Efficacy of diode laser for treating acne keloidalis nuchae. Indian J Dermatol Venereol Leprol. Jan-Feb 2005;71(1):31-4. [Medline].

  20. Califano J, Miller S, Frodel J. Treatment of occipital acne keloidalis by excision followed by secondary intention healing. Arch Facial Plast Surg. Oct-Dec 1999;1(4):308-11. [Medline].

  21. Glenn MJ, Bennett RG, Kelly AP. Acne keloidalis nuchae: treatment with excision and second-intention healing. J Am Acad Dermatol. Aug 1995;33(2 Pt 1):243-6. [Medline].

  22. Bajaj V, Langtry JA. Surgical excision of acne keloidalis nuchae with secondary intention healing. Clin Exp Dermatol. Jan 2008;33(1):53-5. [Medline].

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Numerous acne keloidalis papules and plaques in a white man with straight hair.
A large acne keloidalis plaque in a bandlike distribution at the posterior occiput in an African American man.
A large acne keloidalis plaque on the occipital region in an African American patient. This man also had perifolliculitis of the scalp and acne conglobata (the follicular occlusion triad).
Numerous papules that have coalesced into a large plaque, within which are tufts of hairs with several hair shafts exiting the same follicular orifice.
A dense plasma cell infiltrate surrounding a hair follicle.
Naked hair shafts embedded within a fibrotic dermis.
 
 
 
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